In short: Pulmonary hypertension is high pressure in the lung arteries, which strains the right side of the heart. Its causes fall into five groups, and treatment is determined almost entirely by the cause. The goal of the work-up is therefore not only to confirm the diagnosis, but to identify what is causing it.
Symptoms
Shortness of breath and fatigue on exertion, dizziness or fainting on exertion, chest pressure and leg swelling. The symptoms are non-specific, so the disease is sometimes diagnosed late.
The five groups
- Pulmonary arterial hypertension (PAH): a disease of the small blood vessels of the lung themselves. It can be hereditary, drug-related, or associated with connective tissue disease, liver disease or congenital heart disease, and sometimes has no known cause.
- Due to left heart disease: heart failure or valve disease. This is the most common cause.
- Due to lung disease or low oxygen: COPD, pulmonary fibrosis, sleep apnoea and living at high altitude.
- Obstruction of the lung arteries: mainly CTEPH, in which old blood clots that did not dissolve after a pulmonary embolism turn into scar tissue that blocks the arteries.
- Other or combined causes: blood disorders, sarcoidosis, metabolic diseases, kidney disease and others.
How it is diagnosed
The definitive diagnosis is made by right heart catheterisation. Before catheterisation, a work-up is done to decide whether it is needed and to point to the cause:
- Echocardiography, the main screening test
- Blood tests
- Pulmonary function tests and, when needed, chest CT
- Lung ventilation/perfusion (V/Q) scan, to rule out CTEPH
- Sleep study when needed, to detect sleep apnoea
- Sometimes cardiopulmonary exercise testing (CPET), which we interpret
When needed, the work-up and treatment are carried out in collaboration with a cardiologist.
Treatment
Treatment depends entirely on the cause. In groups 2 and 3, the focus is on treating the underlying heart or lung disease, including oxygen or treatment of sleep apnoea when needed. In PAH there are dedicated drugs that act on the lung blood vessels, and they are usually combined. In CTEPH, alongside long-term anticoagulation, the options include surgery to remove the clots (pulmonary endarterectomy, PEA), balloon pulmonary angioplasty (BPA) and medication. Accurately identifying the group is therefore the basis of every decision.
After a pulmonary embolism
Most patients recover fully. Anyone who still has shortness of breath or reduced exercise capacity after three months of anticoagulant treatment for a pulmonary embolism should be evaluated for CTEPH, a condition with effective treatments, including surgery, balloon angioplasty and medication.
Listen
Living with pulmonary hypertension
Dr. Inbal Shafran in conversation with Shira Emuna Amsalem on the Hebrew-language podcast "Breathing Conversations" (Sichot Neshima) by an Israeli lung patients' association (Lanshom): the path to diagnosis, why it sometimes takes years, and life with a rare disease.
Related topics
General information only; it does not replace a medical consultation. עברית